[HTML][HTML] The transport of low density lipoprotein-derived cholesterol to the plasma membrane is defective in NPC1 cells
KM Wojtanik, L Liscum - Journal of Biological Chemistry, 2003 - ASBMB
Niemann-Pick disease type C (NPC) is characterized by lysosomal storage of cholesterol
and gangliosides, which results from defects in intracellular lipid trafficking. Most studies of
NPC1 have focused on its role in intracellular cholesterol movement. Our hypothesis is that
NPC1 facilitates the egress of cholesterol from late endosomes, which are where active
NPC1 is located. When NPC1 is defective, cholesterol does not exit late endosomes;
instead, it is carried along to lysosomal storage bodies, where it accumulates. In this study …
and gangliosides, which results from defects in intracellular lipid trafficking. Most studies of
NPC1 have focused on its role in intracellular cholesterol movement. Our hypothesis is that
NPC1 facilitates the egress of cholesterol from late endosomes, which are where active
NPC1 is located. When NPC1 is defective, cholesterol does not exit late endosomes;
instead, it is carried along to lysosomal storage bodies, where it accumulates. In this study …